Zollinger-Ellison Syndrome

Published on 19/07/2015 by admin

Filed under Radiology

Last modified 19/07/2015

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 Hypervascular pancreatic mass with multiple peptic ulcers and thickened folds

• Best imaging tool

image Helical CT or MR for pancreas and possible metastasis
image Endoscopic ultrasonography for additional primary sites; guides biopsy

TOP DIFFERENTIAL DIAGNOSES

• Helicobacter pylori gastritis
• Gastric carcinoma
• Gastric metastases and lymphoma
• Extrinsic inflammation
• Other gastritides

PATHOLOGY

• 20-60% of cases are associated with multiple endocrine neoplasia type 1 (MEN1)

CLINICAL ISSUES

• Most common signs/symptoms

image Pain, increased acidity, severe reflux, diarrhea, upper gastrointestinal tract ulcers
image Gastrinomas are often multiple (60%), malignant (60%), and metastatic (30-50%)
• Hypergastrinemia is hallmark of Zollinger-Ellison syndrome (ZES)

image Serum gastrin level of > 1,000 pg/mL is virtually diagnostic of ZES
• Prognosis

image Good with surgical resection of primary gastrinoma
image Poor if gastrinoma, liver metastases, or ulcers recur after surgery
image
(Left) Axial CECT in a 63-year-old man who presented with intractable peptic ulcer disease demonstrates hyperemia and mural thickening image of the stomach.

image
(Right) Arterial phase CECT in the same patient shows a small hypervascular gastrinoma image in the pancreatic head. It is important to distinguish this from the superior mesenteric artery image and superior mesenteric vein image.
image
(Left) Axial CT of a 55-year-old woman with hypercalcemia, diarrhea, and severe abdominal pain as presenting symptoms of MEN1 syndrome shows one of several neck masses image, representing parathyroid adenomas or hyperplasia.

image
(Right) Abdominal CT in the same case shows marked hypervascularity and thickening of the gastric wall image. Multiple liver metastases are present image. The serum gastrin levels were strikingly elevated, confirming ZES, though the gastrinoma was not identified on CT.

TERMINOLOGY

Abbreviations

• Zollinger-Ellison syndrome (ZES)

Definitions

• Severe peptic ulcer disease associated with marked ↑ in gastric acid due to gastrin-producing endocrine tumor (gastrinoma) of pancreas

IMAGING

General Features

• Best diagnostic clue

image Hypervascular pancreatic mass with multiple peptic ulcers and thickened folds
• Location

image Gastrinoma: Pancreas (75%), duodenum (15%), and liver and ovaries (10%)

– Common site: Gastrinoma triangle

image Superiorly: Cystic and common bile ducts
image Inferiorly: 2nd and 3rd parts of duodenum
image Medially: Junction of pancreatic neck and body
image Ulcers: Stomach and duodenal bulb (75%), postbulbar and jejunum (25%)

Radiographic Findings

• Barium studies: Gastric, duodenal, and proximal jejunum

image Large volume of fluid dilutes barium and compromises mucosal coating
image Markedly thickened gastric folds
image Peptic ulcers: Round or ovoid barium collections surrounded by thin or thick radiolucent rim (edematous mucosa) and radiating folds

CT Findings

• Gastrinomas

image Small or large, heterogeneous density lesion, ± cystic and necrotic areas, ± calcification
image Liver metastases are common
image Hypervascular (primary and secondary) lesions ± local or vascular invasion on arterial and portal venous phase
image Inflammatory changes in stomach, duodenum, and proximal small bowel

– Thickened gastric, duodenal, and jejunal folds
image Signs of ulcer penetration

– Wall thickening, luminal narrowing of stomach and duodenum
image Signs of ulcer perforation

– Free air in abdomen (from a duodenal or antral ulcer) or lesser sac (from a gastric ulcer)

MR Findings

• T1WI

image Hypointense pancreatic nodule on fat-saturated sequence
• T2WI

image Hyperintense on spin-echo sequence

– Both primary and metastatic tumors
• T1WI C+

image Hyperintense, hypervascular on fat-saturated delayed spin-echo sequence

Ultrasonographic Findings

• Endoscopic ultrasonography (EUS)

image Detects small gastrinomas better than CT or MR
image Usually homogeneously hypoechoic mass
• Intraoperative ultrasonography

image Detects very small tumors (75-100% sensitivity)

Angiographic Findings

• Conventional

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